TY - JOUR
T1 - Atypical Anti-Glomerular Basement Membrane Nephritis
T2 - A Case Series From the French Nephropathology Group
AU - Chauveau, Bertrand
AU - Gibier, Jean-Baptiste
AU - Olagne, Jérôme
AU - Morel, Antoine
AU - Aydin, Selda
AU - McAdoo, Stephen P
AU - Viallet, Nicolas
AU - Perrochia, Hélène
AU - Pambrun, Emilie
AU - Royal, Virginie
AU - Demoulin, Nathalie
AU - Kemeny, Jean-Louis
AU - Philipponnet, Carole
AU - Hertig, Alexandre
AU - Boffa, Jean-Jacques
AU - Plaisier, Emmanuelle
AU - Domenger, Camille
AU - Brochériou, Isabelle
AU - Deltombe, Clément
AU - Duong Van Huyen, Jean-Paul
AU - Buob, David
AU - Roufosse, Candice
AU - Hellmark, Thomas
AU - Audard, Vincent
AU - Mihout, Fabrice
AU - Nasr, Samih H
AU - Renaudin, Karine
AU - Moktefi, Anissa
AU - Rabant, Marion
AU - CFPR - French Nephropathology Group
PY - 2024
Y1 - 2024
N2 - RATIONALE & OBJECTIVE: Atypical anti-glomerular basement membrane (GBM) nephritis is characterized by a bright linear immunoglobulin staining along the GBM by immunofluorescence without a diffuse crescentic glomerulonephritis nor serum anti-GBM antibodies by conventional ELISA. We characterized a series of patients with atypical anti-GBM disease.STUDY DESIGN: Case series.SETTING & PARTICIPANTS: Patients were identified by the French Nephropathology Group as having atypical anti-GBM nephritis between 2003 and 2022.FINDINGS: Among 38 potential cases, 25 were included. 14 (56%) were female and 23 (92%) had hematuria. Median [interquartile range (IQR)] serum creatinine at diagnosis was 150 [102-203] μmol/L and median [IQR] urine protein to creatinine ratio was 2.4 [1.3-5.2] g/g. 9 (36%) patients had endocapillary proliferative glomerulonephritis (GN), 4 (16%) had mesangial proliferative GN, 4 (16%) had membranoproliferative GN, 2 (8%) had pure and focal crescentic GN, 1 (4%) had focal segmental glomerulosclerosis, and 5 had glomeruli that were unremarkable on histopathology. Nine patients (36%) had crescents, involving a median of 9% of glomeruli. Bright linear staining for IgG was seen in 22 cases (88%) and for IgA in 3 cases (12%). The nine patients (38%) who had a monotypic staining pattern tended to be older with less proteinuria and rarely had crescents. Kidney survival rate at one year was 83% and did not appear to be associated with the light chain restriction.LIMITATIONS: Retrospective case series with a limited number of biopsies including electron microscopy.CONCLUSIONS: Compared to typical anti-GBM disease, atypical anti-GBM nephritis frequently presents with endocapillary or mesangial proliferative glomerulonephritis pattern and appears to have slower disease progression. Further studies are needed to fully characterize its pathophysiology and associated clinical outcomes.
AB - RATIONALE & OBJECTIVE: Atypical anti-glomerular basement membrane (GBM) nephritis is characterized by a bright linear immunoglobulin staining along the GBM by immunofluorescence without a diffuse crescentic glomerulonephritis nor serum anti-GBM antibodies by conventional ELISA. We characterized a series of patients with atypical anti-GBM disease.STUDY DESIGN: Case series.SETTING & PARTICIPANTS: Patients were identified by the French Nephropathology Group as having atypical anti-GBM nephritis between 2003 and 2022.FINDINGS: Among 38 potential cases, 25 were included. 14 (56%) were female and 23 (92%) had hematuria. Median [interquartile range (IQR)] serum creatinine at diagnosis was 150 [102-203] μmol/L and median [IQR] urine protein to creatinine ratio was 2.4 [1.3-5.2] g/g. 9 (36%) patients had endocapillary proliferative glomerulonephritis (GN), 4 (16%) had mesangial proliferative GN, 4 (16%) had membranoproliferative GN, 2 (8%) had pure and focal crescentic GN, 1 (4%) had focal segmental glomerulosclerosis, and 5 had glomeruli that were unremarkable on histopathology. Nine patients (36%) had crescents, involving a median of 9% of glomeruli. Bright linear staining for IgG was seen in 22 cases (88%) and for IgA in 3 cases (12%). The nine patients (38%) who had a monotypic staining pattern tended to be older with less proteinuria and rarely had crescents. Kidney survival rate at one year was 83% and did not appear to be associated with the light chain restriction.LIMITATIONS: Retrospective case series with a limited number of biopsies including electron microscopy.CONCLUSIONS: Compared to typical anti-GBM disease, atypical anti-GBM nephritis frequently presents with endocapillary or mesangial proliferative glomerulonephritis pattern and appears to have slower disease progression. Further studies are needed to fully characterize its pathophysiology and associated clinical outcomes.
U2 - 10.1053/j.ajkd.2023.11.003
DO - 10.1053/j.ajkd.2023.11.003
M3 - Article
C2 - 38171412
SN - 1523-6838
VL - 83
SP - 713-728.e1
JO - American Journal of Kidney Diseases
JF - American Journal of Kidney Diseases
IS - 6
ER -